Anaesthetic Management of A Patient with Sturge-Weber Syndrome Undergoing Oophorectomy

Summary The Sturge-Weber Syndrome (SWS) is a neurocutaneous disorder characterized by leptomeningeal and facial angiomas, neurologicand ocular manifestations. We report a case of oophorectomy for ovarian dermoid in a 14 year-old girl who was a diagnosed case of Sturge-Weber Syndrome. General anaesthesia was given for the procedure. The perioperative anaesthestic management is discussed in the present report.


Introduction
Sturge 1 (1879) first described the clinical features of the disease and Weber 2 (1929) demonstrated the pattern of intracranial calcification and named it EncephalofacialAngiomatosis. TheSturge-Weber Syndrome (SWS) is a rare neurocutaneous syndrome of unknown etiology. It is primarily characterized by congenital cutaneous angiomas, neurologicaland ocular features. 3 LeptomeningealAngiomas may cause hemiparesis, hemianopsia, stroke like episodes, headaches, seizures, developmental delay, learning disabilities and mentalretardation. Vascularangiomas may involve the nose, palate, gingiva, tongue, larynx and trachea posing challenge to laryngoscopy and intubation. Ocular features such as glaucoma make smoothinduction and intubation necessary to limit the rise in intraocular and intracranialpressures. Perioperative hypoxemia, hypoglycemia, hypotension, ischaemiaand hyperthermia may precipitate seizures. The varied presentation and extent, that ranges from localized superficial skin lesions to extensive systemic and airway involvement, makes the anaestheticmanagement challenging. 4

Case report
A 14-year-old girl (53kg), a known case of SWS presented with a lump in lower abdomen since 4 years with progressive increase in size and discomfort. She had recurrent episodes of convulsions, focalwith secondary generalization since 3 months of age with last episode 10 months ago and weakness in right upper extremity since childhood. The patient was on antiepileptic therapy (Carbamazepine 300 mg BD and Clobazam 10mgBD). Examination revealed aleft sided 3 x 3cm port wine stainin V 1 distribution (firstdivision of trigeminalnerve) over forehead, (Fig1 ). Right hemiparesis was also noted. There were no ocular features of SWS or any oropharyngeal hemangiomas on airway evaluation. Routineblood andurine investigations, ECG and chest X-ray were normal. Pelvic ultrasound revealed a 24 x 17 x 9 cm mass arising from the left ovary which was confirmedby CTscan. Alateral skull X-ray revealed gyriform calcifications (Tram-Track appearance) in the left frontoparietal region,(Fig2). CT scan of brain revealed a left parietal angioma with dystrophic cerebralcalcification,(Fig3). MRI scanof brain nyl80 mcg, midazolam 1mg, ranitidine 50 mg and i.v. ondansetron 4 mg 15 minutes prior to induction. Non invasive BP, ECG, SpO 2 and EtCO 2 were used to monitor the vitals. I.V. lidocaine 50 mg was given to attenuate an intubation response. Preoxygenation was followed by induction with propofol 110 mg and vecuronium 5 mg. A careful gentle laryngoscopy and intubation were performed to minimize an intubation response. Anaesthesia was maintained with propofol infusion (100-150mcg.kg -1 .min -1 ), oxygen, nitrous oxideand vecuronium (three intermittent bolus dosesof 1 mgeach at20minuteinterval) withgood haemodynamic stability.Fentanyl 50mcg was repeated intraoperatively. At the endof surgery, the neuromuscular blockade was reversedwith neostigmine 2.5 mgand atropine 1.2 mg. Extubation response was attenuated usinglidocaine 50 mg. The patient wasshifted tothe post anaesthetic care unit (PACU) and post-operaive pain relief was provided with i.v. tramadol50 mgTDS. The recovery was uneventfuland thepatient wasdischarged from the hospital on the 10 th postoperative day.

Discussion
The SWS patients may present for facialand cosmetic surgeries, dental procedures, trabeculectomy, goniotomy, examinationunder anaesthesia,seizure controlsurgery or other surgeries. [4][5][6] Angiomas may involve the airway (nose, palate, gingiva, tongue, larynx andtrachea) leadingto difficult mask ventilation, laryngoscopy and intubation, apart from bleedingcaused by rupture. Intubation should be done using a soft, lubricated, non styleted, cuffed endotracheal tube by expert anaesthesiologist. Because these vessels have abnormal autoregulation, the intraoperative blood pressure should be well controlled. 7 Emotional agitation may increase the volume of haemangiomas markedlyand may necessitate avoiding surgery inthe morning. 5 Vascularchanges may also affect the dura, brain, pituitary, lungs, spleen, and other organs. 4 A thorough evaluation ruledout suchlesions in our patient.Cutaneous featuresinclude PortWine Stain, facialor extrafacial, truncal or on the extremities. Its showed left parietalcerebral calcification, left cerebrocortical atrophy and signs of delayed myelination. Electroencephalography (EEG) showed attenuation of cerebralactivity on theleft side.A therapeutic drug level showed low normal levels of Carbamazepine4 mcg/ml (normal 4-12 mcg/ml) and the dose was increased to 200 mg five times a day and the same dose was continued.
The patientunderwent explorative laparotomy for oophorectomy. She was premedicated with i.v fenta-presence in the V 1 distribution (first division of the trigeminal nerve) is associatedwith ahigher riskof epilepsy or glaucoma. 3 Ocular features include glaucoma (30-71%) most commonly, bupthalmos, choroidal hemangioma, strabismus and others. 3 They may require avoidance of drugs that can cause an increase in intra ocular pressure (IOP) such as succinylcholine and ketamine.Anticholinergics shouldbe avoidedin patients with narrow angle glaucoma.
Cerebral angioma undergoing vascular steal may lead to cerebral ischaemia resulting in intractable and recurrentseizures, status epilepticus, and recurrent vascular events.Seizures are the most common neurologic manifestations, mostly focal, though may be secondarily generalized. Onset of seizures before the age of 2 years suggests a greaterchance ofrefractoriness, mental retardation and neurologic involvement. Earlyonset of seizures, extensive angiomas, progressive neurologic involvement, hemiparesis and deteriorating cognitive functions are predictors of poor outcome 3 . Our patient had an early onset (3 months of age), focal with secondarily genaralised convulsions,with arecurrent course, but they were wellcontrolled withcarbamazepine and clobazam.Preoperatively, antiepileptic therapeutic drug level was done to guide the therapy. Since the therapeutic level of carbamazepine was low, its dose was increased from 600 mg/day to 1000 mg/day. Concommitant chronic use of antiepileptics can affect metabolism of someanaesthetic agents. Low cognitive understandingand mentalretardation caninterfere with doctor-patient communication perioperatively causing emotionalstress, increased blood pressure and consequent swelling of angiomas. It is advisable to interview the patient preoperatively and establish a good rapport. Succinylcholine is avoided in hemiplegia due to its effect on serum potassium 7,8 . We used a fentanylpropofol-vecuronium combinationto achieveand maintain haemodynamic stability intraoperatively 5 . Light plane of anaesthesia, bucking, straining and airway obstruction during induction or emergence can cause an increase in intraocularand intracranialpressures and should be prevented 9 .Events like hypoxemia, hypoglycemia, hypotension, ischaemia and hyperthermia may precipitate status epilepticusand should be avoided 3 .
In case of dentalprocedures with oral angiomas, hemostasismay be difficult to achieve and an overzealous approach should be avoided. Yamashiro and Furara 5 reported cancellation of gingivectomy due to potential for excessive bleeding. The presence of preexistingneurodeficit(right sidedhemiparesis) precluded the use of epidural anaesthesia in our patient. Despite the claims of proconvulsant actions of propofol, there issignificant evidenceto thecontrary. Propofolhas been successfully used to treat status epilepticus 10 and druginduced seizures 11 . Yamashiro and Furura 5 successfully used propofol in a patient of SWS for oral surgery. Cochran et al 12 reported that it is advisable to avoid propofol in patients at risk for seizures and poorly controlled epilepsy. Our patient was wellcontrolled with anticonvulsants, hence we proceeded with the use of propofol. We encountered no problem or complicationswhich can be attributed to carefulscreening, monitoringandanaesthetic management.
A good knowledge andan understandingof SWS, careful preoperative evaluation and a proper anaesthetic management plan are of utmost significance. Achieving control of convulsive d isorder perioperatively,attenuation ofhaemodynamic responses duringairway manipulationand surgery,careful intubation/extubation, avoidingtrauma toangiomatous lesions, limitingthe rise inintra-ocular andintracranialpressures, with adequate pain relief leads to safeanaesthetic management of these patients.